Among the patients who presented to our outpatient clinic, headache (85.7%) and vision defect (32.7%) were the most prevalent symptoms (Table 1). As we observed the cranial magnetic resonance imaging (MRI) findings, the most common findings were sinus vein thrombosis (SVT) (7.1%), posterior optic glob flattening (7.1%) and partial empty sella (4.1%) (Fig. 1). When there is no underlying sinus anomaly or etiology such as SVT in PCS, it is referred to as primary PCS; in contrast, when an identifiable cause is indicated, it is referred to as secondary PCS [7]. In our study there were 35 patients in primary PCS group, 63 patients in secondary PCS group
Table 1
Comparison of gender, etiology (primary and secondary), BMI, and CSF opening pressure with respect to the reason for starting additional medication (topiramate)
| | p |
| Resistance to acetazolamide monotherapy |
Gender | Male | | 18 (48.7) | 0.196(x) |
Female | | 19 (51.4) |
BMI | | 27 [21–30] | 0.008 (xxx) |
CSF opening pressure (cm/H2O) | | 38 [29–50] | 0.325(xxx) |
Etiology | Secondary | | 31 (83.8) | 0.999(xx) |
Primary | | 6 (16.2) |
(x) Pearson’s chi-Squared test was used. Descriptive statistics are presented as numbers (%). |
(xx) Fisher's exact test was used. Descriptive statistics are presented as numbers (%). |
(xxx)Mann–Whitney U test was used. Descriptive statistics are presented as median [interquartile range]. |
P-values indicated in bold were statistically significant (p < 0.05). |
BMI, body mass index; CSF, cerebrospinal fluid |
The relation between SVT presence and mean CSF opening pressure (cm/H2O) in the subjects included in the study was statistically significant (p = 0.011). The CSF opening pressure was higher in cases with SVT (47 cm/H2O) than cases without SVT (31 cm/H2O). Further, although the CSF mean opening pressure was higher in secondary PCS cases than in primary PCS cases, the difference between two groups was not statistically significant (Table 2).
Table 2
Measurement results of RNFL thicknesses according to CSF opening pressures before and after treatment
| | | Pearson's r | p |
CSF opening pressure | - | Nasal RNFL (pretreatment) | 0.282 | 0.025 |
CSF opening pressure | - | Nasal RNFL (posttreatment) | -0.101 | 0.480 |
CSF opening pressure | - | Temporal RNFL (pretreatment) | 0.261 | 0.039 |
CSF opening pressure | - | Temporal RNFL (posttreatment) | -0.173 | 0.225 |
Pearson’s correlation coefficient was used. |
CSF, cerebrospinal fluid; RNFL, retinal nerve fiber layer |
There was a statistically significant, linear, unidirectional, and weak correlation between CSF opening pressure and the number of unloading LPs. In other words, as the CSF opening pressure increased, the need for repetitive unloading LPs also increased.
As we know up to 15% of weight loss associated with PCS remission [8], first of all obese patients are referred to a hospital-based weight management programme. As the first line medical therapy, acetazolamide dose was 10–20 mg/kg/day. Treatment efficiency was evaluated by repetitive (every 3 months) OCT and visual field examinations. We administered topiramate (1–3 mg/kg/day) as a second line medical therapy to patients who had acetazolamide side effect (intolerable metabolic acidosis, 4 male, 10 female patients) or had resistance to acetazolamide monotherapy.
Monotherapy resistance was observed in patients (18 male, 19 female patients) with high body mass index (BMI). A statistically significant relation was found between high BMI and the requirement for additional medication (topiramate) owing to drug resistance developed against the first-line acetazolamide therapy (It was not significant in the group who ceased acetozalamide treatment due to acidosis side effect (4 male, 10 female) and switched to topiramate treatment). When other comparisons were examined, no statistically significant differences were noted (Table 3). During the treatment, mean headache relief time was 30 days, mean vision defect relief time was 60 days. On follow up, mean drug withdrawal time was 6 months and mean follow up time was 1.8 years.
Table 3
Comparison of RNFL thicknesses before and after treatment
| Pretreatment | Posttreatment | p |
Nasal RNFL | 521.59 ± 157.97 | 425.88 ± 107.02 | < 0.001 |
Temporal RNFL | 738.41 ± 165.27 | 625.57 ± 118.33 | < 0.001 |
Dependent samples t test was used. |
RNFL, retinal nerve fiber layer |
Objective and quantitative diagnostic and follow-up criteria are needed because patient compliance is not always optimal in the evaluation of visual acuity and visual field in the pediatric population, possible refractive errors may affect visual acuity, and the pediatric population gets bored and easily distracted during the examination. Although disc appearance gives an idea about the differential diagnosis of papilledema in fundoscopy, OCT is an objective, rapid, quantitative, and reproducible imaging method in the differential diagnosis of pseudopapilledema causes such as small optic disc appearance or optic disc drusen and in monitoring treatment response. The results of our study further prove OCT as a useful method (Fig. 2).
When the CSF opening pressure measured by LP performed for diagnosis was compared with the nasal and temporal RNFL values obtained via OCT, a statistically significant increase was found in RNFL values as the CSF opening pressure increased. However, the difference in posttreatment values was not significant (Table 4).
As we compared the pre- and posttreatment OCT values, the difference between the mean nasal and temporal RNFL values before and after the treatment was statistically significant (Table 5).
The mean pre- and posttreatment nasal and temporal RNFL values were evaluated according to the etiology of the cases (primary and secondary cases). Pretreatment RNFL thickness was relatively greater in secondary cases compared with that in primary cases, but the difference between the mean values was not statistically significant.
When the pre- and posttreatment BMI and nasal and temporal RNFL values were compared, no statistically significant differences were noted (p > 0.05 for each). In fact, RNFL values were lower in obese patients.